A cross-sectional nationwide survey on esophageal atresia and tracheoesophageal fistula.

BACKGROUND: Our study aims at disclosing epidemiology and most relevant clinical features of esophageal atresia (EA) pointing to a model of multicentre collaboration. METHODS: A detailed questionnaire was sent to all Italian Units of pediatric surgery in order to collect data of patients born with EA between January and December 2012. The results were crosschecked by matching date and place of birth of the patients with those of diagnosis-related group provided by the Italian Ministry of Health (MOH). RESULTS: A total of 146 questionnaires were returned plus a further 32 patients reported in the MOH database. Basing on a total of 178 patients with EA born in Italy in 2012, the incidence of EA was calculated in 3.33 per 10,000 live births. Antenatal diagnosis was suspected in 29.5% patients. 55.5% showed associated anomalies. The most common type of EA was Gross type C (89%). Postoperative complications occurred in 37% of type C EA and 100% of type A EA. A 9.5% mortality rate was reported. CONCLUSIONS: This is the first Italian cross-sectional nationwide survey on EA. We can now develop shared guidelines and provide more reliable prognostic expectations for our patients.

[Congenital deformities of the chest wall. Surgical treatment]. / Le malformazioni congenite della parete toracica. Trattamento chirurgico.

BACKGROUND AND AIMS: Malformations of the front chest wall are congenital defects that have been reported since the seventeenth century and which include the clinical syndromes of funnel chest, pigeon chest and Poland's syndrome. Although they have been reported for such a long time, their pathogenesis is in many ways still unknown and the theories proposed up until now reveal uncertain and unsatisfactory findings. Attempts to gather precise information regarding their real incidence are equally ineffective given that frequently those cases which are not particularly severe are not referred for specialist care. These malformations usually involve severe psychological problems caused by the deformity and, in more severe cases, may lead to alterations in normal cardiac and respiratory function which are above all evident during intense physical effort. METHODS: The authors critically review the cases of chest malformation corrected by the Department of Pediatric Surgery at the G. Gaslini Institute during the period from 1986 to date. They describe the surgical techniques used, justifying their choice and discussing any postoperative complications. RESULTS: This experience shows that there is a clear preference for less invasive surgery for the correction of funnel chest compared to the technique proposed by Ravitch in which the sternum was totally mobilised following its extensive detachment from the other bone and muscular structures of the thoracic cage, with a high risk of damaging the internal mammary artery. The operation that was felt to be most suitable for correcting this defect was that described by Wesselhoett and De Luca in 1982. It is easier to perform and less invasive: it eliminates the detachment phase by inserting a support in titanium alloy through the sternal frame. Autologous grafts taken from the bottom contralateral ribs to the defect were used to treat Poland's syndrome in line with the technique suggested by Ravitch, thus achieving good stability of the whole chest; in view of the young age of the patients undergoing correction, it was not thought appropriate to proceed with the cosmetic reconstruction of the pectoral muscles using a peduncled flap of latissimus dorsi. A total of 70 patients were operated in this way; the cosmetic results were very satisfactory, whereas complications were limited to 5 cases of intraoperative pneumothorax, 5 cases of serohematic subcutaneous collection which were treated conservatively, and 1 case of hypertrophic scarring.